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Query: UMLS:C0018681 (headache)
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The authors report a rare case of a huge jugular foramen neurinoma extending to pharyngeal region in a 64 year-old woman who visited an otolaryngologist with complaints of dysphagia, hoarseness and headache in 1984. At that time a submucosal lump was noted in her left pharyngeal region. Biopsy of the tumor proved it to be neurinoma. A CT scan disclosed a dumbell shaped jugular foramen neurinoma and noncommunicating hydrocephalus. Because her symptoms slowly progressed and cerebellar signs as well as signs of increased intracranial pressure was noted, she was referred to our hospital in 1986. She had shown typical sings of left Vernet syndrome, VIIth, VIIIth cranial nerve impairment as well as cerebellar, long tract and increased ICP signs. A subtotal removal was performed from the extracranial and posterior fossa in one stage. The tumor seemed to originate from the Xth cranial nerve. The histological diagnosis of neurinoma was confirmed. Postoperatively, although her dysphagia increased for several weeks, a tracheostomy was not necessary. She was discharged 2 months later and returned to her usual occupation as a house wife. Slight dysphagia and hoarseness were her only chronic symptoms. So far, 88 cases of jugular foramen neurinoma have been reported, 15 of them including our case from Japan. Our case is probably the largest of these reported tumors. Tumors in jugular foramen often masquerade as an acoustic neurinoma or other tumor. However, recently this can usually be diagnosed preoperatively with a careful neurological examination and by means of neuroradiological investigations such as jugular venography, CT scan and MRI.
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PMID:[Huge jugular foramen neurinoma extending to the pharyngeal region: a case report]. 341 64

A 40-year-old man was admitted to our department, because of sudden onset of dysphagia, hoarseness, left neck pain and headache. There were no skin lesions. On neurological examination, there were paralysis of the left soft palate and constrictor muscles of the pharynx, weakness of the left sternocleidomastoid and left upper trapezius. In cerebrospinal fluid (CSF) examination, cell count and protein concentration were elevated. Antibody titer to varicella zoster virus (VZV) was elevated in both the serum and CSF. And VZV-DNA was detected by PCR from CSF. Gd enhanced MRI showed the nodular lesion at the left jugular foramen. The diagnosis of Vernet's syndrome (VS) associated with VZV infection was made. The patient's symptoms were immediately improved with 30 mg of prednisone and 3 g of varaciclovir daily for 14 days. Only a few cases of VS due to VZV have been reported previously. Our case is the first case that detected VZV-DNA in CSF by PCR.
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PMID:A case of Vernet syndrome with varicella zoster virus infection. 1845 82

Vernet syndrome is a unilateral palsy of glossopharyngeal, vagus, and accessory nerves. Varicella zoster virus (VZV) infection has rarely been described as a possible cause. A 76-year-old man presented with 1-week-long symptoms of dysphonia, dysphagia, and weakness of the right shoulder elevation, accompanied by a mild right temporal parietal headache with radiation to the ipsilateral ear. Physical examination showed signs compatible with a right XI, X, and XI cranial nerves involvement and also several vesicular lesions in the right ear's concha. He had a personal history of poliomyelitis and chickenpox. Laringoscopy demonstrated right vocal cord palsy. Brain MRI showed thickening and enhancement of right lower cranial nerves and an enhancing nodular lesion in the ipsilateral jugular foramen, in T1 weighted images with gadolinium. Cerebrospinal fluid (CSF) analysis disclosed a mild lymphocytic pleocytosis and absence of VZV-DNA by PCR analysis. Serum VZV IgM and IgG antibodies were positive. The patient had a noticeable clinical improvement after initiation of acyclovir and prednisolone therapy. The presentation of a VZV infection with isolated IX, X, and XI cranial nerves palsy is extremely rare. In our case, the diagnosis of Vernet syndrome as a result of VZV infection was made essentially from clinical findings and supported by analytical and imaging data.
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PMID:Vernet syndrome resulting from varicella zoster virus infection-a very rare clinical presentation of a common viral infection. 2953 42

This case describes a 60-year-old immunosuppressed man after renal transplant who presented to the emergency department with 1 week of generalized weakness, a 20-lb unintentional weight loss, sore throat, dysarthria, dysphagia, cough, and shortness of breath. Additionally, he developed tinnitus, headaches, photophobia, and neck stiffness. He underwent an extensive workup including a lumbar puncture with meningitis and encephalitis panel, which was positive for varicella zoster virus. He never developed a dermatomal vesicular rash but had persistent dysphagia and aspiration and was eventually diagnosed with Vernet syndrome. This case highlights theories for the increase in varicella zoster virus encephalitis cases causing neurologic symptoms and proposes that this trend is likely to continue.
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PMID:Cranial nerve involvement in varicella zoster virus after renal transplantation. 3310 May 43